title: Childhood interstitial lung diseases: an 18-year retrospective analysis.
abstract: OBJECTIVE
  Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns.
  
  
  METHODS
  Childhood ILD cases seen at Vanderbilt Children's Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system.
  
  
  RESULTS
  A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review.
  
  
  CONCLUSIONS
  Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy.
authors.affiliation: Division of Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, 2200 Children's Way, 11215 Doctor's Office Tower, Nashville, TN 37232-9500. lisa.young@vanderbilt.edu.
authors.forename: Jennifer J
authors.initials: JJ
authors.lastname: Soares
authors.forename: Gail H
authors.initials: GH
authors.lastname: Deutsch
authors.forename: Paul E
authors.initials: PE
authors.lastname: Moore
authors.forename: Mohammad F
authors.initials: MF
authors.lastname: Fazili
authors.forename: Eric D
authors.initials: ED
authors.lastname: Austin
authors.forename: Rebekah F
authors.initials: RF
authors.lastname: Brown
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Sokolow
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Hilmes
authors.forename: Lisa R
authors.initials: LR
authors.lastname: Young
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
keywords: childhood lung disease; interstitial lung disease; lung biopsy